The immune system is the front-line defense mechanism of the body that fights infections and is the key to our overall health. A weak immune system will result in frequent infections, allergies, growth failure and many other systemic features. The immune system is very complex and comprises of cells, proteins and small molecules that are scattered in the body in a complex fashion and work synchronously to protect the body against any foreign threat. The threat may come from outside i.e. in the form of infections or may arise within body itself, e.g. in the case of cancers.
When a child is born, most of the immune defense is provided by the antibodies passed down by the mother during birth and through breastfeeding. His immune system starts developing after six months of age. It matures around adolescence. We commonly see that infections are much more common in children (esp. early school going children). Most vaccination is also given during early childhood to protect children against lethal infections. In early childhood, upto 5-6 episodes of upper respiratory tract infections and 4-5 episodes of diarrhea in a year are considered normal. These are actually important for building up the immune system later on.
Immunology related problems in children include problems due to increase as well as decrease in immunity. Not only poor immune activity causes infections, even heightened immune response can cause problems that we will be discussing subsequently.
Immunodeficiency diseases
When the body’s defense mechanism is not working properly, the person becomes susceptible to many infections. Mild forms of immunodeficiency disorders may go unnoticed till adult stage, while some severe forms become symptomatic within the first year of life and need to be picked up early.
Immunodeficiencies can be categorized as Primary and Secondary.
Primary Immunodeficiency disorders (PIDs) – These are disorders that are inherited right at birth (genetic origin) and cause some severe problems with the immune system. Some of these disorders are very severe (eg. Severe combined immunodeficiency (SCID)) and affected children catch infections right at birth. Because of completely non-functional immune system, these children do not respond to the standard antibiotics and eventually die if not given a specialized therapy early. Other PIDs are relatively less severe and manifest a little later in life (some even in adolescence or adulthood).
Primary Immunodeficiency can present with symptoms like ear infections, bronchitis, pneumonia, sinus infections, autoimmune disorders, skin disorders, blood disorders like low platelet count or anemia, digestive problems, delayed growth/ development, meningitis , blood malignancy or lymphoma, recurrent abortions, inflammation and infection of internal organs. Immunodeficiencies do not only manifest as infections but these patients can also have multiple allergies, rash, lymphoproliferation (enlargement of liver, spleen, lymph nodes) and even malignancies.
Secondary Immunodeficiency diseases– The immune system becomes weak due to long term medicines like long term steroids, immunosuppressive therapy, chemotherapy and following procedures like bone marrow transplant and solid organ transplants.
Most of these conditions are treatable if diagnosed early. There is a general lack of awareness among the physicians about the existence of these disorders. Most of them either don’t know about these conditions, or believe that these diseases are incurable.
Diagnosis of immunodeficiency disorders
Some PIDs can even be picked at bedside without requiring much investigations, e.g. congenital neutropenia. The diagnosis involves a detailed medical history, physical examination and a set of specialized investigations. Blood tests like immunoglobulin profile, lymphocyte subset are easily available in most major cities. Some investigations are highly specialized and need advanced machinery, but they are also available at various government funded research centers at a very subsidized rate (even free for poor patients).
Treatment
Treatment depends on the type and severity of the immunodeficiency. The ones with severe immunodeficiency (SCID) need to be picked up very early in life and offered a bone marrow transplant (BMT). The longer they go without a BMT, the more are their chances of catching a life-threatening infection. These days BMT can be performed at a subsidized rate through crowd funding and philanthropic organizations. In other less severe immunodeficiency states children are given monthly immunoglobulin therapy and prophylactic antibiotics. Many of these children lead an infection free life and continue to live normally. The milder diseases may only need prophylactic antibiotics to stay free from infections.
It is also important to do prenatal counseling for families who have one affected child with a genetic form of immunodeficiency. The risk of recurrence of genetic diseases in the next child of parents who already have an affected child ranges from 25-50%. These disorders can be picked up in the fetus during early pregnancy. So it is very important to counsel the parents of an affected child for their future pregnancies.
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10 Warning Signs of Primary Immunodeficiency in children
1. Four or more new ear infections within 1 year .
2. Two or more serious sinus infections within 1 year.
3. Two or more pneumonias within 1 year .
4. Two or more months on antibiotics with little effect
5. Failure of an infant to gain weight or grow normally .
6. Recurrent , deep skin or organ abscesses .
7. Two or more deep-seated infections including septicemia .
8. Persistent thrush in mouth or fungal infection on skin.
9. Need for intravenous antibiotics to clear infections .
10. A family history of Primary Immunodeficiency
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10 warning signs of immunodeficiency in adults:
1. > 2 ear infections in one year
2. > 2 sinus infections in one year in the absence of allergies
3. 1 pneumonia per year for more than one year
4. Chronic diarrhea with weight loss
5. Repeat viral infections (colds, herpes, warts, condyloma)
6. Recurrent need for intravenous antibiotics to clear infections
7. Recurrent, deep abscesses of the skin or internal organs (ex. : liver, lungs)
8. Persistent thrush or fungal infection on skin or elsewhere
9. Infection with normally harmless tuberculosis-like bacteria
10. Family history of a primary immunodeficiency
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